2: Vascular Disorders

Question 1

A generalized bleeding that is seen in acquired or congenital defects in secondary hemostasis

Answer 1

Anatomic hemorrhage

Question 2

Hemorrhagic spot, 1 cm or larger in diameter typically forming an irregular blue or purplish patch. Also known as bruise

Answer 2

Ecchymoses

Question 3

Nosebleed that requires intervention

Answer 3

Epistaxis

Question 4

Bleeding from multiple sites, spontaneous and recurrent bleeds or hemorrhage that requires physical intervention of transfusion

Answer 4

Generalized hemorrhage

Question 5

Chronic joint bleed that cause inflammation and immobilization

Answer 5

Hemarthroses

Question 6

Vomiting of bright red blood

Answer 6

Hematemesis

Question 7

Localized collection of extravasated blood, usually clotted, in an organ space or tissue

Answer 7

Hematoma

Question 8

Intact RBCS in the urine

Answer 8

Hematuria

Question 9

Free hemoglobin in the urine

Answer 9

Hemoglobinuria

Question 10

Expectoration of blood secondary to hemorrhage in the larynx, trachea, bronchi, and lungs

Answer 10

Hemoptysis

Question 11

Acute severe blood loss requiring intervention and transfusion

Answer 11

Hemorrhage

Question 12

Containing dark red or black blood

Answer 12

Melena stool

Question 13

Abnormally heavy or prolonged menstrual periods

Answer 13

Menorrhagia

Question 14

A generalized bleeding that may indicate a disorder of primary hemostasis

Answer 14

Mucocutaneous hemorrhage or systemic bleeding

Question 15

Pinpoint purple or red spots on the skin or mucous membranes, approx 1 mm in diameter

Answer 15

Petechiae

Question 16

Purple skin discoloration, typically rounded with a diameter of greater than 3 mm

Answer 16

Purpura

Question 17

Formation, development, or presence of a clot in a blood vessel

Answer 17

Thrombosis

Question 18

Hereditary vascular disorders:

Answer 18

1. Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)
2. Hemangioma-thrombocytopenia syndrome (Kasabach-Merritt syndrome)
3. Ehlers-Danlos syndrome

Question 19

Acquired vascular disorders:

Answer 19

1. Allergic purpura (Henoch-Schonlein purpura)
2. Parapoteinemia and Amyloidosis
3. Senile purpura
4. Drug-induced vascular purpuras
5. Vitamin C deficiency (Scurvy)

Question 20

Most common inherited disorder

Answer 20

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

Question 21

Characterized by a thin-walled blood vessels with discontinuous endothelium, inadequate smooth muscle, and inadequate/missing elastin in the surrounding stoma

Answer 21

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

Question 22

Wall of the affected blood vessels are thin and lack of smooth muscle

Answer 22

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

Question 23

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) universal finding

Answer 23

Epistaxis (nosebleed)

Question 24

Disorders associated with tumors composed of blood vessels that commonly swell and bleed at the surface

Answer 24

Hemangioma-thrombocytopenia syndrome (Kasabach-Merritt syndrome)

Question 25

Manifested by hyperextensive skin, hypermobile joints, joint laxity, fragile tissues, bleeding tendency, and primarily subcutaneous hematoma formation

Answer 25

Ehlers-Danlos Syndrome

Question 26

Connective tissue elastic fibers in small arteries are calcified and structurally abnormal

Answer 26

Pseudoxanthoma Elasticum

Question 27

Subarachnoid and gastrointestinal bleeding are the most common causes of death

Answer 27

Pseudoxanthoma Elasticum

Question 28

Characterized by skeletal and ocular defects

Answer 28

Marfan syndrome

Question 29

More appropriately applied when the conditions is an acute IgA-mediated disorder

Answer 29

Henoch-Schönlein Purpura

Question 30

Universal sign of Allergic purpura (Henoch-Schonlein purpura)

Answer 30

Reddish-purple spots

Question 31

Primarily a disease of children (3-7) years old

Answer 31

Allergic purpura (Henoch-Schonlein purpura)

Question 32

Platelet function can be inhibiting by myeloma proteins

Answer 32

Paraproteinemia

Question 33

Common in elderly men due to lack of collagen support for small blood vessels

Answer 33

Senile purpura

Question 34

Most frequent drugs in drug-induced vascular purpura

Answer 34

Sulfonamides Iodides

Question 35

ROW syndrome mode of inheritance

Answer 35

Autosomal dominant

Question 36

Lesions blanch when pressure is applied

Answer 36

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)

Question 37

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome) usually manifests by

Answer 37

Puberty

Question 38

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome) type of mutations:

Answer 38

Type 1: ENG gene Type 2: ACRVL1 gene Type 3: Unknown

Question 39

Juvenile Polyposis/HHT is caused by mutations in the gene

Answer 39

SMAD4

Question 40

Diagnosis of Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)

Answer 40

- Spontaneous recurrent epistaxis - cutaneous telangiectasia - visceral involvement - familial heredity

Question 41

Associations of Kasabach-Merritt Syndrome:

Answer 41

- Giant Cavernous Hemangioma (Vascular tumor) - Thrombocytopenia - Bleeding Diathesis

Question 42

Complications of Kasabach-Merritt Syndrome:

Answer 42

- microangiopathic hemolytic anemia - sequestration of platelets in hemangiomas - acute or chronic DIC

Question 43

Can be ascribed to defects in collagen production, structure, or cross-linking, with resulting inadequacy of the connective tissues

Answer 43

Ehlers-Danlos Syndrome

Question 44

Mode of inheritance of EDS

Answer 44

Autosomal dominant

Question 45

Greater risk for major bleeding of EDS

Answer 45

EDS IV

Question 46

EDS IV is a structural defect in proa1 (III) chain of collagen encoded by

Answer 46

COL3A1 gene

Question 47

Pseudoxanthoma Elasticum is also known as

Answer 47

Groenberg-Stranberg Syndrome

Question 48

Pseudoxanthoma Elasticum mode of inheritance

Answer 48

Autosomal recessive

Question 49

Pseudoxanthoma Elasticum mutations in the gene

Answer 49

ABC-C6 gene

Question 50

The skin lesions are urticarial and gradually becomes pinkish, then red, and finally hemorrhagic

Answer 50

Henoch-Schenlein Purpura

Question 51

Severe hemorrhagic manifestations as a result of combination of hyperviscosity and platelet dysfunction

Answer 51

Dysproteinemia

Question 52

Do not blanch with pressure

Answer 52

Senile purpura